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To characterise the clinical features of human leucocyte antigen (HLA)-B27+acute anterior uveitis (AAU) patients with or without ankylosing spondylitis (AS) and investigate the retinal vascular involvement in these patients.A total of 1056 HLA-B27+ AAU patients (1525 eyes) were retrospectively studied from April 2008 to February 2016. Patients were divided into human leucocyte antigen (HLA)-B27+AS+ and HLA-B27+AS− group. Clinical features including the onset of uveitis, laterality, the age at first attack, clinical examinations, best corrected visual acuity (BCVA), abnormalities in fundus fluorescence angiography (FFA) and complications were determined and compared between these two groups.There were 581 (55.0%) and 475 (45.0%) patients respectively classified into HLA-B27+AS+ and HLA-B27+AS− group. Males had a higher prevalence than females in the HLA-B27+AS+ group (75.2%) as compared with the HLA-B27+AS− group (51.8%, p<0.001). The HLA-B27+AS+ patients showed a higher percentage of bilateral/alternating involvement (47.3%) as compared with the HLA-B27+AS− group (36.6%, p=0.001). A higher percentage of fibrinous exudation, synechiae as well as complications including complicated cataract and secondary glaucoma were found in the HLA-B27+AS+ group as compared with the HLA-B27+AS− group. Worse visual outcome as indicated by a higher percentage of patients with BCVA <0.5 and with BCVA <0.05 was noted in the HLA-B27+AS+ group as compared with the HLA-B27+AS− group both before and after treatment. FFA showed mild capillary fluorescence leakage in the late phase with indistinctly defined margins on the peripheral retina in 39.3% of HLA-B27+ AAU patients. There was no difference concerning the retinal vascular involvement between these two groups.Our study confirmed that HLA-B27+AS+ patients show a higher percentage of males, more common bilateral involvement, a higher frequency of fibrinous exudates, synechiae and secondary glaucoma as compared with HLA-B27+AS− patients. Visual outcome was poorer, possibly due to the higher prevalence of complicated cataract in HLA-B27+AS+ patients. Retinal vascular involvement was not uncommon in HLA-B27+ AAU patients.